Cysteine and methionine catabolism (WP4504)
Homo sapiens
This pathway visualizes the conversion of methionine to cysteine, after which it is further metabolized in either an oxidative or non-oxidative manner. Several of these metabolites are altered in people suffering from MoCD and SO; changes in metabolite concentrations (comparing MoCD cases to controls) are highlighted in blue (see [https://www.wikipathways.org/index.php/Pathway:WP4507] for the pathway on MoCD). This pathway was inspired by Chapter 12 of the book of Blau (ISBN 3642403360 (978-3642403361)).
Authors
Denise Slenter , Egon Willighagen , Irene Hemel , Daniel Domingo-Fdez , Friederike Ehrhart , Finterly Hu , and Eric WeitzActivity
Discuss this pathway
Check for ongoing discussions or start your own.
Cited In
Are you planning to include this pathway in your next publication? See How to Cite and add a link here to your paper once it's online.
Organisms
Homo sapiensCommunities
Inherited Metabolic Disorders (IMD) Pathways Rare DiseasesAnnotations
Pathway Ontology
methionine cycle/metabolic pathway amino acid metabolic pathway cysteine and methionine metabolic pathway cysteine metabolic pathway disease pathway methionine degradation pathway| Label | Type | Compact URI | Comment |
|---|---|---|---|
| Methionine | Metabolite | chebi:57844 | |
| Tetra-hydrofolate | Metabolite | chebi:57453 | |
| Cystathionine | Metabolite | chebi:58161 | |
| -CH3 | Metabolite | chebi:32875 | |
| ATP | Metabolite | chebi:30616 | |
| Sulfate | Metabolite | chebi:16189 | |
| KG | Metabolite | chebi:16810 | aka a-ketoglutarate (a-KG) |
| Taurine | Metabolite | chebi:15891 | |
| 3-Mercaptopyruvic acid | Metabolite | chebi:57678 | |
| Sulfite | Metabolite | chebi:17359 | |
| S-Adenosylmethionine | Metabolite | chebi:59789 | |
| Cysteine | Metabolite | chebi:35235 | |
| CO2 | Metabolite | chebi:16526 | |
| SO3 2- | Metabolite | chebi:17359 | AKA sulfite ion. |
| Glutathione | Metabolite | chebi:57925 | |
| S-Adenosylhomocysteine | Metabolite | chebi:57856 | |
| Dimethylglycine | Metabolite | chebi:58251 | |
| 5-Methyltetrahydrofolate | Metabolite | chebi:18608 | |
| Hypotaurine | Metabolite | chebi:57853 | |
| Gamma-glutamylcysteine | Metabolite | chebi:58173 | |
| Cystine | Metabolite | chebi:17376 | |
| H2S | Metabolite | chebi:29919 | aka Hydrogen sulfide |
| H2O | Metabolite | chebi:15377 | |
| S-sulfocysteine | Metabolite | chebi:27891 | |
| Betaine | Metabolite | chebi:17750 | |
| Alpha-KB | Metabolite | chebi:16763 | |
| Pyr | Metabolite | chebi:15361 | AKA Pyruvic acid |
| Cysteine sulfinic acid | Metabolite | chebi:61085 | |
| Serine | Metabolite | chebi:33384 | |
| Glu | Metabolite | chebi:29985 | AKA glutamine |
| O2 | Metabolite | chebi:15379 | |
| Thiosulfate(2−) | Metabolite | chebi:16094 | AKA thiosulfate ion |
| B-Sulfinyl pyruvate | Metabolite | hmdb:HMDB0002332 | |
| Homocysteine | Metabolite | chebi:58199 | |
| RSSH | Metabolite | chebi:30605 | AKA persulfurated thiol-containing compound (RSSH) |
| H2S | Metabolite | chebi:16136 | aka Hydrogen sulfide |
| Adenosine | Metabolite | chebi:16335 | |
| NH4+ | Metabolite | chebi:28938 | |
| Glu | Metabolite | chebi:28300 | AKA glutamine |
| Gly | Metabolite | chebi:57305 | AKA glycine |
| MAT | GeneProduct | uniprot:Q00266 | aka Methionine adenosyltransferase |
| SO | GeneProduct | uniprot:P51687 | AKA Sulfite oxidase |
| MT | GeneProduct | brenda:2.1.1 | AKA Methyltransferase |
| SO | GeneProduct | brenda:1.8.3.1 | AKA Sulfite oxidase |
| GS | Protein | uniprot:P48637 | AKA Glutathione synthetase |
| MS | Protein | uniprot:Q99707 | AKA Methionine synthase |
| MPST | Protein | uniprot:P25325 | AKA 3-mercaptopyruvate sulfurtransferase |
| GCS | Protein | uniprot:Q14TF0 | AKA Glutamate-cysteine ligase |
| SAAH | Protein | uniprot:P23526 | AKA Adenosylhomocysteinase |
| CDO | Protein | uniprot:Q16878 | Cysteine dioxygenase type 1 |
| CSD | Protein | uniprot:Q9Y600 | AKA Cysteine sulfinic acid decarboxylase |
| CSE | Protein | uniprot:P35520 | aka Cystathionine beta-synthase |
| AAT | Protein | uniprot:A0A140VK69 | AKA Aspartate aminotransferase |
| BHMT | Protein | uniprot:Q93088 | AKA Betaine--homocysteine S-methyltransferase 1 |
| CSE | Protein | uniprot:P32929 | Cystathionine gamma-lyase |
| SQR | Protein | uniprot:Q9Y6N5 | AKA quinone oxidoreductase |
| AAT | Protein | uniprot:P17174 | AKA Aspartate aminotransferase |
| SQR | Protein | uniprot:Q9Y6N5 | |
| SDO | Protein | uniprot:O95571 | AKA sulfur dioxygenase |
References
- Physician’s Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases [Internet]. Blau N, Duran M, Gibson KM, Dionisi-Vici C. Springer; 2014. 0 p. Available from: https://books.google.com/books/about/Physician_s_Guide_to_the_Diagnosis_Treat.html?hl=&id=wJRBnwEACAAJ OpenLibrary Worldcat
- Protonation state of methyltetrahydrofolate in a binary complex with cobalamin-dependent methionine synthase. Smith AE, Matthews RG. Biochemistry. 2000 Nov 14;39(45):13880–90. PubMed Europe PMC Scholia
- Domain alternation switches B(12)-dependent methionine synthase to the activation conformation. Bandarian V, Pattridge KA, Lennon BW, Huddler DP, Matthews RG, Ludwig ML. Nat Struct Biol. 2002 Jan;9(1):53–6. PubMed Europe PMC Scholia
- Dissecting the catalytic mechanism of betaine-homocysteine S-methyltransferase by use of intrinsic tryptophan fluorescence and site-directed mutagenesis. Castro C, Gratson AA, Evans JC, Jiracek J, Collinsová M, Ludwig ML, et al. Biochemistry. 2004 May 11;43(18):5341–51. PubMed Europe PMC Scholia
- Contribution of cysteine aminotransferase and mercaptopyruvate sulfurtransferase to hydrogen sulfide production in peripheral neurons. Miyamoto R, Otsuguro KI, Yamaguchi S, Ito S. J Neurochem. 2014 Jul;130(1):29–40. PubMed Europe PMC Scholia